In this guide, we explore what causes scleroderma, what the symptoms of scleroderma are, and what treatments are available. If you live with scleroderma, remember that you are not alone and there are many sources of support available to you and your family.

Any medical information provided here is for informational purposes and does not replace medical advice given to you by a medical professional. If you’re concerned that you may have scleroderma symptoms, please see your GP.

What is scleroderma?

Scleroderma is an incurable autoimmune disease which causes skin and tissue around the body to thicken. There are two main types of scleroderma; the mildest form called localised scleroderma, and a more serious form called systemic sclerosis, which may affect the tissues of the internal organs in addition to the skin.

Only around 19,000 people in the UK have a scleroderma diagnosis, which makes it quite uncommon. Scleroderma usually affects people between the ages of 25 and 55, but it is not unheard of in children. Scleroderma is much more common in women than men.

Scleroderma can lead to disability and, depending on which type a person has, the condition can be life-limiting if organs are affected. However, there are treatments to help relieve symptoms of scleroderma and enable those with the condition to live well.

What causes scleroderma?

The immune system is a network of cells and organs in the body that work together to fight off infections, bacteria and organisms to keep the body healthy. An autoimmune disease occurs when the immune system mistakenly attacks the body itself, such as tissues, joints, bones, or organs. This immune system dysfunction is what causes scleroderma, but nobody knows for sure why this occurs for some people and not others. It’s thought that genetics may be involved, and you are more likely to develop scleroderma if a family member has the disease. A person who has one autoimmune condition is also more likely to develop another autoimmune condition, for example, there is a link between Raynaud’s disease and scleroderma.

What are the different types of scleroderma?

Scleroderma is experienced differently by each person with the condition. There are different types of scleroderma, and the type that a person is diagnosed with is likely to affect the symptoms of scleroderma they have and the prognosis of the disease. Sometimes, the milder type of scleroderma can develop into the more serious type.

Below are the different scleroderma types, the symptoms that are likely to be linked to each, and the differences between the two main types.

Localised scleroderma

This type of scleroderma causes hard skin, which may gradually begin to occur in one place and then, in other areas of the body. There are different subtypes of localised scleroderma too, and they’re defined by the areas of skin that are affected, and how. For example, plaque morphea scleroderma causes patches of skin to become reddish brown. Localised scleroderma doesn’t usually lead to complications, although the skin may be uncomfortable, unsightly and painful.

Limited scleroderma (also called systemic scleroderma) and diffuse scleroderma

These differ from localised scleroderma because additional symptoms may occur. Both may also affect the functioning of internal organs, leading to other health conditions developing, or long-term health problems. Limited scleroderma affects certain areas of the body, whereas diffuse scleroderma affects the whole body.

What are the symptoms of scleroderma?

Everyone experiences scleroderma differently. How scleroderma affects a person’s daily life will depend on the type of scleroderma they have, if their treatment is effective, and how severe their symptoms are. Here we list some of the symptoms associated with the two main types of scleroderma. These symptoms are also present in other health conditions.

If you think you may have any of the scleroderma symptoms listed here, talk to your GP.

Localised scleroderma symptoms include:

• Patches of skin that may be itchy, look shiny, or discoloured.
• Areas of thickened skin on the face, head, legs or arms may cause growth or mobility problems if bone and muscles are affected.

Systemic scleroderma symptoms may include:

• Thickening of the skin anywhere on the body, as well as odd spots and lumps
• Infections of the skin
• Skin ulcers
• Organs and the digestive system more likely to be affected
• Swallowing difficulties
• Heartburn
• Raynaud’s disease
• Fatigue
• Joint pain, inflammation, swelling, stiffness due to the skin and tissue around joints becoming thickened and hard
• Weight loss
• High blood pressure
• Pulmonary hypertension

Diagnosis of scleroderma

Scleroderma can be difficult to diagnose. Some GPs rarely see the condition, but they’re likely to refer you to a consultant, who will likely be a rheumatologist initially. The thickening of skin is often the key to a diagnosis but other tests may be performed, such as a blood test for autoantibodies, which could suggest the person has an overactive immune system, as seen in scleroderma.

Living with scleroderma

Learning that you have scleroderma can be difficult and receiving a diagnosis can lead to many concerns. You may never have heard of this condition and be worried about what the future may hold for you, and how it will affect your daily life.

However, many people live well with scleroderma and their prognosis is good. Scleroderma is likely to affect a person’s life in various ways, depending on their symptoms, and which type of scleroderma they have. Here, we list some of the challenges that a person living with scleroderma may face and ways they can help to combat these head-on.

Physical appearance

Because scleroderma causes changes to the skin, such as tightening and thickening, it can change appearance. Some people with the condition may be conscious of this if the skin is visible to other people. There are creams and treatments to help skin look and feel better. There are also ways to cover areas of skin that people are conscious of, using camouflaging make-up and other products.

Lifestyle

People living with scleroderma can do most of the things that other people do, such as go on holidays, work and enjoy a social life, but symptoms may affect these activities. It is advisable for people living with scleroderma to have as healthy lifestyle as possible in order to retain their general health. Some people may develop coeliac disease, so may require a gluten-free diet. Getting regular exercise is also important as well as quitting smoking and reducing high blood pressure.

Fatigue

A common symptom for several reasons, scleroderma causes fatigue which can be difficult to manage and may affect everyday life. Fatigue may be due to medication side effects, using lots of energy due to having weakened muscles, difficulties sleeping at night, and pain. Generally, people experiencing fatigue are advised to spread activities out across the day and week, ensuring they have frequent periods of rest, rather than doing too much at once and feeling exhausted.

Sex

Most men and women living with scleroderma are able to have sex and enjoy sex, but some may have issues if scleroderma affects their genitals, or if they develop depression, anxiety or health conditions. There are lots of ways to help with sexual health difficulties though and these should be discussed with your GP in the first instance.

Pregnancy

Lots of women living with scleroderma are able to have children but some may have increased risks to themselves or their baby.

Prognosis and survival

Living with a chronic and life-limiting health condition inevitably brings anxiety about the future and what this will hold. Scleroderma is unlikely to be life-limiting for them. However, for others with systemic scleroderma, which affects internal organs, they may be living with the prospect that their condition may progress more severely and affect how long they live. This can be a difficult thing to deal with, but there are lots of support services that can provide help for scleroderma patients in dealing with the unknown aspects of their condition.

Talk to your GP or scleroderma specialist if you are finding it hard to manage concerns about prognosis and survival.

What type of treatments are available for scleroderma?

For some people, scleroderma may get better on its own, depending on the type of condition they have, but systemic scleroderma may progress over time. There are a variety of treatments for scleroderma which may be available, such as drug medications, localised or topical treatments, therapeutic programmes like physiotherapy, and many self-care treatments.

The scleroderma treatments listed below are for some of the more common symptoms. This is not an exhaustive list, and if you are finding any of your symptoms difficult to manage, it is advisable to talk to your GP or specialist team.

• Skin soreness, ulcers, tightening, dryness - Keeping skin moisturised may provide some relief while physiotherapy can help with tight skin.
• Raynaud’s - Try to stay as warm as possible by wrapping up in cold weather, wearing extra pairs of gloves and socks, using heat pads, and keeping your house warm.
• Digestion - People living with scleroderma may have digestive or gastrointestinal symptoms such as diarrhoea, gas, bloating, constipation, heartburn, weight loss, poor appetite. There are medications that can be used and lots are available over the counter.
• High blood pressure - This can be treated with lifestyle changes and medication such as ACE inhibitors.
• Joint pain and inflammation - Pain relief and anti-inflammatory drugs can be used to treat this symptom.
• Stress, anxiety and depression - Issues such as these are common for people who are living with a health condition and there are lots of ways to help, including anti-depressant medication and psychological therapy.

Products for scleroderma

Daily living aids are products designed to help you with mobility, both around the home and outdoors, getting dressed and doing general daily tasks.

At Complete Care Shop, we’re experts in daily living aids. If you have scleroderma, you may find a rollator helpful for getting about the house and heading to the shops. When relaxing at home, a rise and recline chair can help with getting up and sitting down. When it comes to preparing food at home, our specially-made utensils and perching stools make life a little easier.

Diet and exercise for scleroderma

A nutritional diet is important for anybody living with scleroderma. People with systemic sclerosis are likely to have digestive issues and may experience weight loss as a result of their condition, so eating a balanced diet is essential. It is important to get enough calcium, vitamin D, iron and healthy fats. Fibre can help with blood pressure and anti-inflammatory foods, such as ginger, may help reduce some symptoms.

If a person has coeliac disease as well as scleroderma, they may need to follow a gluten-free diet.

Some people take vitamins and minerals as supplements for scleroderma, such as vitamins A, C, D, E, K, folate, B12, zinc and iron. Some people take probiotics to help with digestive issues, or anti-oxidant and anti-inflammatory supplements like turmeric, selenium and beta-carotene. It’s advisable to discuss this with your GP before taking scleroderma supplements.

In terms of exercise, it’s important for people living with scleroderma. Although it may feel challenging for some people, for example, if their skin is tight and uncomfortable. Any activity that gets the body moving, within the reasonable limits, is beneficial for joints, muscles, lungs, heart and mind. It’s important for people living with scleroderma to obtain advice before starting an exercise programme from their specialist team or a physiotherapist.

How can scleroderma affect employment?

Lots of people who live with scleroderma are able to work. Some people who have systemic sclerosis that progresses to a more serious stage may decide to leave work if their symptoms make it difficult to work.

Support and resources for people living with scleroderma

We hope that our scleroderma information guide has proved helpful. Scleroderma is not generally very well understood so can be challenging for someone who receives a diagnosis.

It’s key to remember, though, that there are a number of support options available to you. Some people find it helps to talk to others who are affected by scleroderma, so we’ve listed some online support groups. We also have provided links to other online resources with information and advice. If you’re concerned about anything you have read in this guide, please discuss with your GP.

Communities

• Scleroderma and Raynaud’s UK online community - a forum for people affected by Raynaud’s or scleroderma, to share experiences.
• Scleroderma Facebook group - an online support group for scleroderma patients and their families.

Resources

• Ceoliac UK - for those living with scleroderma who also have coeliac disease, Coeliac UK provides a wealth of information about the condition.
• NHS - source of official medical information about causes, symptoms and treatments for Raynaud’s and related conditions.
• PHA UK - a charity supporting people who have pulmonary hypertension, which may be a complication of systemic sclerosis.
• Scleroderma and Raynaud’s UK - a charity providing support and information to the millions of people living with scleroderma, Raynaud’s and associated conditions, with lots of information about daily life.

Scleroderma glossary

Although we always try to explain things as simply and as clearly as possible, it’s occasionally necessary to use the correct medical terminology. These medical terms are often known for being tricky to pronounce and understand. So, we’ve put together a list of terms used on this page along with a brief explanation of what they mean to help your understanding of scleroderma.

• Inhibitors – medication used to treat high blood pressure which blocks the body producing angiotensin II – a chemical that narrows blood vessels.
• Autoimmune diseases – conditions where the immune system attacks the body, mistaking it for an invading virus or bacteria.
• Coeliac disease – digestive health condition caused by the immune system reacting to a protein called gluten which is found in wheat, barley and rye, and then attacking the tissues of the gut, causing intestinal damage and an array of unpleasant symptoms.
• Pulmonary hypertension – a condition caused by high blood pressure that affects the pulmonary arteries, damaging them and affecting blood supply to the lungs and heart.
• Rheumatologist – a doctor specialising in conditions affecting the musculoskeletal system. For example, bones and tissue.